Full Workup Research Articles

T2 Hypointense Schwannoma Masquerading As A Vascular Lesion: A Case Report.

Spinal schwannomas are rare nerve root tumors that typically produce subtle symptoms from nerve root compression. These tumors are known to be heterogeneously hyperintense on T2-weighted MRI and avidly enhance with contrast. However, here we describe an L5 spinal schwannoma in a 66-year-old woman that was hypointense on T2-weighted imaging with other radiographic findings more closely aligning with a vascular lesion. The neuroradiologic characteristics on MRI, time resolved MR angiography, and catheter digital subtraction angiography are presented. The patient underwent a full workup for possible dural arteriovenous fistula and thrombosed venous varix before the nerve sheath tumor was discovered intraoperatively and confirmed as a schwannoma on histopathology.

Accuracy of pelvic measurements on virtual radiographic projections based on computed tomography scans compared to conventional radiographs pre- and postoperatively

BackgroundThe anteroposterior (ap) radiograph of the pelvis is decisive in the diagnosis of different pathologies of the hip joint. Technical advantages have reduced the radiation dose of pelvic CT to levels comparable to radiographs. The purpose of this study was to validate if standard radiographic parameters (lateral center edge angle, medial center edge angle, acetabular index, acetabular arc, extrusion index, crossover sign and posterior wall sign) can accurately be determined on radiograph-like projections reconstructed from the CT dataset pre- and postoperatively.MethodsA consecutive series of patient with symptomatic dysplasia of the hip and a full radiologic workup (radiographs and CT scan pre- and postoperatively) who underwent periacetabular osteotomy were included. Standard radiographic parameters were compared between radiographs and radiograph-like projections by two authors pre- and postoperatively.ResultsA total of 16 hips (32 radiographs/32 radiograph-like projections) were included in the study. No significant difference was found between the radiographs and radiograph-like images for all parameter for both examiners. ICC between radiograph and radiograph-like projections for all investigated parameters showed good to excellent reliability (0.78–0.99) pre- and postoperatively.ConclusionRadiograph-like projections show comparable results to radiographs with regard to the important investigated parameters (lateral center edge angle, medial center edge angle, acetabular index, acetabular arc, extrusion index, crossover sign and posterior wall sign). Thus, ultra-low-dose CT scans may reduce the need for conventional radiographs in pre- and postoperative analyses of 3-dimensional hip pathologies in the future, as the advantages increasingly outweigh the disadvantages.

Autism spectrum disorder in the fragile X premutation state: possible mechanisms and implications.

There is increasing recognition of the heterogeneity of origin of cases of autism spectrum disorder (ASD) with multiple forms of ASD having been identified over the decades. Among these, a genetic etiology can be identified in 20-40% of cases when a full genetic work-up is completed. The Fragile X premutation state (characterized by the presence of 55-200 CGG repeats in the FMR1 gene) is a relatively newly identified disease state that has since been associated with several disorders including fragile X-associated tremor ataxia syndrome (FXTAS), fragile X-associated primary ovarian insufficiency (FXPOI) and most recently, fragile X-associated neurodevelopmental disorders (FXAND) which commonly includes anxiety and depression. In addition to these associated disorders, extant literature and clinical observations have suggested an association between the premutation state and ASD. In this paper, we review the literature pertinent to this and discuss possible molecular mechanisms that may explain this association. This includes lowered levels of the FMR1 Protein (FMRP), GABA deficits, mitochondrial dysfunction and secondary genetic abnormalities that is seen in premutation carriers as well as their increased vulnerability to environmental stressors. Understanding these mechanisms can facilitate development of targeted treatment for specific sub-groups of ASD and premutation disorders in future.

Psychosis as the Initial Presenting Symptom of Anti-Hu Encephalitis: A Case Series with Literature Review

IntroductionAnti-Hu related Paraneoplastic Neurological Syndrome (PNS) is one of the most common paraneoplastic-associated neurological syndromes (Kayser 2010). While the primary clinical manifestations are neurologic in nature (Smitt 2002), only rare reports exist regarding psychiatric manifestations. Our poster presents two cases of Anti-Hu Encephalitis manifesting as psychosis as well as a systematic literature review on the co-occurrence of psychosis and PNS.ObjectivesThe aim of this case series is to show psychosis as the primary symptom of a paraneoplastic syndrome that does not typically present in this way. It also serves as a reminder to have a detailed work-up and maintain a wide differential diagnosis when evaluating patients with first-episode psychosis.MethodsTwo cases of anti-Hu encephalitis primarily presenting with psychiatric symptoms are discussed. A systematic literature review was carried out based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) model on three electronic databases: PubMed, Embase, and PsycINFO. Search terms included were (Anti-Hu) AND (Psychosis OR Hallucinations OR Schizophrenia OR Schizoaffective).ResultsOur case series reports on two patients with diagnosed anti-Hu encephalitis who were treated by our psychiatry team, where the primary manifestations of the illness were psychiatric in nature. Psychotic symptoms in these cases were managed with Risperidone, Olanzapine, and Paliperidone.ConclusionsPsychotic symptoms are seldom reported in the literature and cases like the ones presented emphasize the importance of a full medical work-up for first episode psychosis as well as a wide differential. Given the increased association between PNS and psychiatric illness, more emphasis and further research is warranted.DisclosureNo significant relationships.

Diagnosing dementia in the Arctic: translating tools and developing and validating an algorithm for assessment of impaired cognitive function in Greenland Inuit

IntroductionThe ageing Arctic populations raise the need for work-up of cognitive function that reflects language and cultural understandings.ObjectivesTo translate and evaluate tools for work-up of cognitive impairment in Greenland.MethodsStep A: An expert panel was established to select tools suitable for the work-up of cognitive impairment at three different settings in Greenland. Step B: Tools were translated in a multiple-step process of independent translations with back-translation and adaptations by two independent translators and two Greenlandic physicians. Step C: a testing and validation process of the tools at three locations: the national hospital in the capital city; regional hospital in a town; health care centre in a small town.ResultsTools selected were Mini-Cog and RUDAS. Participants for testing of tools were 43 of 61 invited, of which six had dementia. RUDAS and Mini-Cog scores were associated (p < 0.001). The smoothed AUC was 0.87 (95%-CI, 0.65–0.95) for Mini-Cog and 0.90 (95%-CI, 0.76–0.97) for RUDAS. The sensitivity of Mini-Cog with a cut-off at ≤3 was 83.3%, and specificity was 62.2%. For RUDAS with a cut-off at ≤23, these were 100% and 75.7%, respectively.ConclusionsRequested tools have been translated for assessing cognitive function in the native Arctic setting. Small town residents with a Mini-Cog score of 3 or lower should be referred to a regional hospital for RUDAS, and a score of 23 or less should cause referral to the national hospital for a full work-up of cognitive function.DisclosureNo significant relationships.

PD14-03 ANALYSIS OF TWO INDEPENDENT RISK STRATIFICATION EVALUATION TOOLS IN A COHORT OF PATIENTS WITH MICROSCOPIC HEMATURIA

You have accessJournal of UrologyCME1 May 2022PD14-03 ANALYSIS OF TWO INDEPENDENT RISK STRATIFICATION EVALUATION TOOLS IN A COHORT OF PATIENTS WITH MICROSCOPIC HEMATURIA Thomas Williams, Eric Macdonald, Max Kuster, Michelle Ou, Tavya Benjamin, Manish Vira, and Simon Hall Thomas WilliamsThomas Williams More articles by this author , Eric MacdonaldEric Macdonald More articles by this author , Max KusterMax Kuster More articles by this author , Michelle OuMichelle Ou More articles by this author , Tavya BenjaminTavya Benjamin More articles by this author , Manish ViraManish Vira More articles by this author , and Simon HallSimon Hall More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000002546.03AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: While the prevalence of microscopic hematuria (MH) in the general population remains high, the malignancy detection rate is low. Extensive workup may create unnecessary radiation and instrumentation. Multiple guidelines and nomograms have been published to help reduce excessive evaluations. This study aimed to review our institution’s malignancy detection rate, modelled using the revised 2021 AUA/SUFU Microhematuria guideline and Kaiser-Permanente Hematuria Risk Index (HRI). METHODS: Retrospective review of our microscopic hematuria database of 3,147 patients to analyze malignancy detection rate, stratified for the 2021 AUA guidelines and HRI risk categories. Patient inclusion was RBC ≥ 3/hpf with completed fields for demographics, risk factors, and full work-up. Categorization of malignancy was further broken down into grade, stage, and type. Solid parenchymal tumors (SPT) were included if pathologically confirmed or highly suspicious on imaging. Patients who presented with MH but had a history of gross hematuria were stratified per AUA or HRI grouping. Statistical analysis was performed by area under receiver operating characteristic curves. RESULTS: 2,001 patients met inclusion criteria. Overall, 36 neoplasms were diagnosed after workup: 21 non-muscle invasive bladder cancer, 1 muscle-invasive bladder cancer, 2 upper tract urothelial cancers, 12 SPTs. AUA risk categories had a total positive detection rate of 0% (Low), 0.67% (Medium), and 2.83% (High). HRI score (0-11) showed malignancy detection rates of 0.97% (Low; 0-4), 2.65% (Moderate; 5-8), and 9.68% (High; 9-11). Looking only at urothelial malignancies (excluding SPT), the breakdown (AUA versus HRI respectively) was Low: 0 vs 0.18%, Medium/Moderate: 0.13 vs 2.28%, and High: 2.10 vs 9.68%. The HRI classified 1,139 patients as low-risk with 2 urothelial malignancies detected (0.18%), while the AUA classification only included 163 patients (0%). The HRI score stratification urothelial detection area under the curve for ROC was 0.812 compared to 0.709 (AUA). CONCLUSIONS: After stratifying our database population based on the 2021 AUA/SUFU Guideline and Kaiser-Permanente HRI, we found that both groups had linear increases in malignancy detection rates with increasing risk group. However, the HRI, which uses a more granular categorization, would have avoided unnecessary workup in a significantly higher proportion of patients. Future directions should analyze further sub-stratification to create a more specific nomogram that avoids unnecessary workup. Source of Funding: None © 2022 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 207Issue Supplement 5May 2022Page: e257 Advertisement Copyright & Permissions© 2022 by American Urological Association Education and Research, Inc.MetricsAuthor Information Thomas Williams More articles by this author Eric Macdonald More articles by this author Max Kuster More articles by this author Michelle Ou More articles by this author Tavya Benjamin More articles by this author Manish Vira More articles by this author Simon Hall More articles by this author Expand All Advertisement PDF DownloadLoading ...

V13-12 SINGLE PORT DA VINCI ROBOTIC RADICAL ADRENALECTOMY VIA MCBURNEY INCISION

You have accessJournal of UrologyCME1 May 2022V13-12 SINGLE PORT DA VINCI ROBOTIC RADICAL ADRENALECTOMY VIA MCBURNEY INCISION Jason Elyaguov, Mubashir Billah, Michael Stifelman, and Mutahar Ahmed Jason ElyaguovJason Elyaguov More articles by this author , Mubashir BillahMubashir Billah More articles by this author , Michael StifelmanMichael Stifelman More articles by this author , and Mutahar AhmedMutahar Ahmed More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000002646.12AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Incidental adrenal masses are often benign and biopsy is not recommended. However, malignant potential exists in 6% of lesions >4cm and as high as 25% in lesions over 6cm, as a result surgical resection is recommended for masses over 5cm. Robotic resection today is considered standard of care, and the single port approach is novel and minimally described. In this video, we aim to review da Vinci single port (SP) access for left adrenalectomy via a McBurney incision as well as describe robotic instrument and assistant port configuration via the specialized da Vinci SP globe and demonstrate surgical technique for efficient and safe adrenal gland excision. METHODS: 59 year old male initially presented with left flank pain and CT imaging showed large left renal cysts as well as an incidental heterogenous 4.8 cm x 3.9 cm left adrenal mass with mixed attenuation. Full metabolic workup was normal. Surveillance MRI showed the mass to be >5cm and therefore patient elected for surgical resection. He underwent an SP left adrenalectomy in the right lateral decubitus position via a McBurney incision employing the specialized multi-access SP globe port, allowing for optimal dissection. We highlight efficient and judicious use of bipolar cautery in ligating the adrenal vein and excising out the adrenal gland. RESULTS: The patient tolerated the procedure well with a total operative time of 78 minutes and minimal blood loss. No intra-abdominal drain was placed, while a Foley catheter was removed 12 hours post operatively. The patient was discharged within 24 hours with no residual symptoms or complications at 90-day follow-up. The small single incision healed very well. Final pathology favored adrenal adenoma with a 4.2cm tumor and positive-free margins. CONCLUSIONS: SP da Vinci robotic adrenalectomy with multi-access globe port is feasible and safe. The McBurney incision allows for efficient access to the renal hilum and avoids risk of midline hernia, while providing excellent cosmesis and minimal pain. Source of Funding: None © 2022 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 207Issue Supplement 5May 2022Page: e1034 Advertisement Copyright & Permissions© 2022 by American Urological Association Education and Research, Inc.MetricsAuthor Information Jason Elyaguov More articles by this author Mubashir Billah More articles by this author Michael Stifelman More articles by this author Mutahar Ahmed More articles by this author Expand All Advertisement PDF DownloadLoading ...

An Update on Neurosurgical Management of Primary CNS Lymphoma in Immunocompetent Patients.

Primary central nervous system lymphomas (PCNSL) are rare CNS tumors that harbor a conspicuously longer diagnostic delay compared to other malignant brain tumors. The gold standard for diagnosis is stereotactic biopsy to acquire tissue for histopathological analysis and therefore neurosurgery plays a central role when reducing the diagnostic period is mandated. However, histopathological diagnosis could be complicated if the patient was preoperatively exposed to corticosteroids. Besides the histopathological result, diagnosis of a PCNSL also requires full diagnostic workup to exclude cerebral metastatic disease of a systemic lymphoma. Most reviews of PCNSL discuss recent advancements in systemic treatment options from an (neuro-)oncologic viewpoint, whereas our intention was to discuss the optimization of the diagnostic period and therefore describe current standards of imaging, summarizing the diagnostic workup, discussing the surgical workup and future diagnostic prospects as well as the influence of preoperative corticosteroid therapy to reduce the diagnostic delay of PCNSL patients.

MRI and rectal endoscopy sonography performance to diagnose the digestive depth infiltration of pelvic endometriosis.

The main objective of this study was to evaluate the performances of MRI and rectal endoscopy sonography (RES) in predicting the depth of bowel wall infiltration by deep infiltrating endometriosis (DIE). We conducted a single center retrospective study from April 2014 to March 2020 including all patients who had undergone digestive tract resection (discoid or segmental) for DIE removal and who had benefited from full preoperative imaging workup based on both pelvic MRI and RES. Fifty two patients were enrolled in the study. Median age was 35.8years (26.1-44.5years). Indications for surgery mainly comprised chronic pelvic pain (94.2%) and infertility (36.5%). Overall, pathological examination showed digestive involvement in 92.3% of patients, while transmural infiltration was found in 38.4% of cases. In contrast, both MRI and RES suspected transmural involvement in 42 patients (80.8%). Corresponding sensitivity and specificity were 0.95 [95% CI (0.751-0.999)] and 0.28 [95% CI (0.137-0.467)], respectively. Our results revealed agreement between MRI and RES in 85% of cases with a kappa at 0.5 [95% CI (0.207-0.803), moderate agreement]. Subgroup analysis in patients with transmural MRI lesions showed a sensitivity of 0.95 [95% CI (0.740-0.999)] and a specificity of 0.13 [95% CI (0.028-0.336)]. Our study suggests that performing a second-line examination is not useful if there is no transmural impairment in MRI or RES. Nevertheless, the combination of these two preoperative examinations seems to be essential for the evaluation of the depth of digestive involvement of endometriosis to guide surgical management as effectively as possible. The constitution and training of multidisciplinary expert groups must be developed to be able to offer optimal patient management.

Assessment for residual disease after pulmonary endarterectomy in patients with chronic thromboembolic pulmonary hypertension.

ObjectivesPulmonary endarterectomy (PEA) is recommended for eligible patients with chronic thromboembolic pulmonary hypertension (CTEPH) and is potentially curative. However, persistent/recurrent CTEPH post-PEA can occur. Here we describe symptom and diagnostic assessment rates for residual disease post-PEA and longitudinal diagnostic patterns before and after riociguat approval for persistent/recurrent CTEPH after PEA.MethodsThis US retrospective cohort study analysed MarketScan data (1 January 2002–30 September 2018) from patients who underwent PEA following a CTEPH/pulmonary hypertension (PH) claim with at least 730 days of continuous enrolment post-PEA. Data on pre-specified PH symptoms and the types and timings of diagnostic assessments were collected.ResultsOf 103 patients (pre-riociguat approval, n=55; post-riociguat approval, n=48), residual PH symptoms >3 months after PEA were reported in 89% of patients. Overall, 89% of patients underwent one or more diagnostic tests (mean 4.6 tests/patient), most commonly echocardiography (84%), with only 5% of patients undergoing right heart catheterisation (RHC). In the post- versus pre-riociguat approval subgroup, assessments were more specific for CTEPH with an approximately two-fold increase in 6-min walk distance and N-terminal prohormone of brain natriuretic protein measurements and ventilation/perfusion scans, and a four-fold increase in RHCs.ConclusionsLow RHC rates suggest that many patients with PH symptoms post-PEA are not being referred for full diagnostic workup. Changes to longitudinal diagnostic patterns may indicate increased recognition of persistent/recurrent CTEPH post-PEA; however, there remains a need for greater awareness around the importance of continued follow-up for patients with residual PH symptoms post-PEA.

Incidence of Deep Vein Thrombosis in Cerebral Palsy Following an Orthopaedic Surgical Event.

The purpose of this study was to identify the incidence of venous thromboembolism (VTE) and characterize the demographics, comorbidities, and risk factors for patients with cerebral palsy (CP) having orthopaedic surgery. All patients diagnosed with CP who underwent an orthopaedic surgical procedure at one institution between 2008 and 2017 were identified. Diagnosis codes and associated patient events were recovered from the electronic medical record. Each VTE event was reviewed to ascertain an actual VTE episode related to a surgical event. The review included 2583 orthopaedic surgical events in 1371 patients. Of the initial 88 cases identified, 28 cases had a deep thrombosis documented. Six cases of VTE occurred within 3 months following the surgical event. Three of these cases had thigh thrombosis, and 2 patients had upper arm thrombosis, and 1 patient had a superior vena cava thrombosis. On further workup, 5 of these 6 patients were identified as having a congenital hypercoagulable condition. VTE is a relatively rare occurrence after orthopaedic surgery in pediatric patients with CP, but when it occurs, a full hematologic workup for a congenital hypercoagulable condition is indicated. Based on the low incidence of thigh thrombosis, routine pharmacological or intermittent mechanical calf compression is not recommended. A careful clinical and family history should be performed to identify patients with possible genetic hypercoagulable conditions who would merit prophylaxis. Level IV.

Metastatic Breast Cancer Simulating Cirrhosis with Portal Hypertension and Acute Liver Failure: A Case of “Pseudocirrhosis”

Pseudocirrhosis describes morphological changes in the liver that resemble cirrhosis, but the histopathological findings are not suggestive of cirrhosis. It occurs most often in patients with metastatic breast cancer, although it has also been reported in other malignancies. As in cirrhosis, portal hypertension is often seen in patients with pseudocirrhosis. Pseudocirrhosis is a rare but important complication of metastatic cancer. We will describe the case of a 45-year-old woman with a history of breast cancer, without any previously known liver disease presenting with subacute liver failure. Initial imaging studies indicated cirrhosis of the liver without signs of focal malignancy. Full diagnostic workup was negative for specific causes of liver disease and provided no evidence of tumor recurrence. Malignant pseudocirrhosis is an unusual type of metastatic tumor, with spread representing a rare but important differential diagnosis of progressive liver failure.

Clinical outcomes after refixation of subacute repaired distal biceps tendon ruptures

BackgroundThe aim of this study was to evaluate the clinical outcome and complication spectrum after delayed repair surgery of distal biceps tendon ruptures, postulating that satisfactory results are possible contrary to previous literature.MethodsForty-three of 92 patients with a full workup (= OPT in) undergoing primary distal biceps tendon repair were included in this study. The mean age of participants was 49.5 years (range = 22-66 years). This cohort was divided into two groups: patients undergoing acute repair (<21 days = AR group) and a group with delayed intervention (>21 days = SR group). Beside clinical evaluation, functional scores and detection of heterotopic ossification were documented. Strength of flexion and supination were measured using a BIODEX multipoint system. In addition, thirty-one patients were included only in the evaluation of complications in the absence of consent for clinical examination (= OPT out).ResultsConcerning the OPT-in group, twenty-eight patients (ø age = 48.9 years; 22-63 years) received acute repair after an average of 9.2 ± 3.7 days. On the contrary, 15 patients (ø age = 50.5 years; 32-66 years) were treated with a delay after an average of 31.4 ± 10.4 days. Regarding patient-reported outcome measures, conflicting results emerge (AR/SR: Subjective Elbow Value = 87/80%, P > .05; Mayo score = 96/93 pts, P > .05; the Disabilities of the Arm, Shoulder and Hand score = 6/13 pts, P < .05; and Oxford Elbow Score = 44/39 pts, P < .05). The main complication is the paresthesia of the lateral antebrachial cutaneous nerve, which occurs more in the group of delayed repair (AR: 21.0%, SR 31.8%). Forty-one percentage of patients in the SR group described pain in the elbow with exertion in contrast to 17.3% in the AR group. In terms of elbow strength, no significant difference in the AR or SR group compared with the contralateral side could be observed.ConclusionThe data suggest that delayed repair of distal biceps ruptures beyond 3 weeks may result in satisfactory clinical outcomes. However, exertional pain and paresthesia of the lateral antebrachial cutaneous nerve may diminish results.

Bilateral lipid keratopathy with rheumatic fever in siblings.

To report two unusual cases of lipid keratopathy in siblings with rheumatic fever and positive anti-phospholipid antibodies. Clinical and histopathological examination along with complete systemic work-up was done after taking appropriate consent from the patients. A serum lipid profile showed elevated cholesterol and low-density lipoproteins. Systemic history revealed both the siblings were on treatment for rheumatic fever. A complete blood profile showed positive anti-phospholipid antibodies in one of them. Penetrating keratoplasty was performed in both cases one year apart. Histopathological examination showed lipid filled macrophages distributed throughout the corneal stroma. No episode of recurrence was noted in the follow-up period of three years. These two cases highlight the importance of a thorough family history and a full systemic work-up in a patient with bilateral symmetrical lipid keratopathy. The co-existence of rheumatic fever and anti-phospholipid syndrome has been studied in literature but, their presence along with lipid keratopathy has not been previously described. It requires further research whether they have an association or it was just an incidental finding.

Liver manifestations and complications in inflammatory bowel disease: A review.

Hepatobiliary manifestations are common in inflammatory bowel disease (IBD), with 30% of patients presenting abnormal liver tests and 5% developing chronic liver disease. They range from asymptomatic elevated liver tests to life-threatening disease and usually follow an independent course from IBD. The pathogenesis of liver manifestations or complications and IBD can be closely related by sharing a common auto-immune background (in primary sclerosing cholangitis, IgG4-related cholangitis, and autoimmune hepatitis), intestinal inflammation (in portal vein thrombosis and granulomatous hepatitis), metabolic impairment (in non-alcoholic fatty liver disease or cholelithiasis), or drug toxicity (in drug induced liver injury or hepatitis B virus infection reactivation). Their evaluation should prompt a full diagnostic workup to identify and readily treat all complications, improving management and outcome.

Alcohol Withdrawal Induced Malignant Catatonia and Response to Bromocriptine: Case Report

Catatonia and malignant catatonia may result in devastating and life-threatening complications like pulmonary embolisms, pneumonia, deep venous thrombosis, rhabdomyolysis, and even death. There have been documented cases implicating alcohol withdrawal as a significant culprit in catatonia. Here, we provide a unique case report of a patient with a complicated medical course, who subsequently developed malignant catatonia secondary to severe alcohol withdrawal, and was successfully treated using both first line treatment of catatonia (lorazepam), and second-line treatment (bromocriptine). Mr. KR is a 32 year-old male with a psychiatric history significant for severe alcohol use disorder, developed fevers, rigidity and dysarthria throughout his admission despite a full negative infectious workup. He was intubated twice, he received his first doses of bromocriptine 2.5mg BID on day 29 of hospitalization. On day 30, he was extubated, and by day 31 he was afebrile, his rigidity and dysarthria had subsided, and he was able to converse coherently. Further titration of bromocriptine (up to 2.5mg every six hours on days 33-37) showed continued improvement, and the patient was eventually transferred out of the ICU. On day 37, a bromocriptine wean was initiated, which KR tolerated and showed continued improvement with return of some baseline activities and resolution of dysarthria. This case report demonstrates the need for increased suspicion for alcohol withdrawal catatonia in patients with a complicated course of alcohol withdrawal, and illustrates a previously undocumented etiology for malignant catatonia.

Glial Fibrillary Acidic Protein (GFAP) Astrocytopathy Presenting as Mild Encephalopathy with Reversible Splenium Lesion

IntroductionAutoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is scarce and usually presents as meningoencephalomyelitis. Here, we offer the case of an atypical presentation of GFAP-astrocytopathy.Case PresentationWe report the case of a 26-year-old woman admitted to our neurology department for a 3-week progressive and worsening neurologic picture, with secondary worsening. Initial imaging showed a Mild Encephalitis with Reversible Splenium of corpus callosum lesion (MERS). Full infectious and autoimmune workup then revealed positivity of GFAP antibodies, leading us to diagnose GFAP astrocytopathy.DiscussionOur case is the first reported association between MERS and GFAP astrocytopathy in an adult patient. Clinical presentation of GFAP astrocytopathy usually includes various neurologic symptoms and can lead to misdiagnosis.

Lip angioedema after indirect contact with a sea anemone.

Sea anemones are marine animals that can produce toxins causing severe angioedema. Swimmers and divers should be aware of sea anemone species that can cause local and systemic toxic reactions and avoid indirect or direct skin contact. High index of suspicion, full laboratory workup, and treatment with steroids and antibiotics are imperative for an uneventful recovery.

Dermatologic manifestations of pediatric cardiovascular diseases: Skin as a reflection of the heart.

Cutaneous disease can often be an initial clue of an underlying cardiovascular disease. Many congenital conditions (ie, Noonan syndrome with multiple lentigines, Carney complex, and Fabry disease) and acquired conditions may present initially with specific cutaneous features that should prompt clinicians to conduct a full cardiac workup. Given the extensive number of conditions with both cardiovascular and cutaneous findings, this review will focus on diseases with cardiocutaneous pathology with hopes of raising clinician awareness of these associations to decrease morbidity and mortality, as several of these diseases often result in fatal outcomes.

Histopathology, salivary flow and ultrasonography of the parotid gland: three complementary measurements in primary Sjögren's syndrome.

ObjectiveThe involvement of salivary glands in primary SS (pSS) can be assessed in different ways: histopathology, salivary flow and ultrasonography. To understand the relative value of these different approaches, it is crucial to understand the relationship between them. As we routinely perform these three modalities in the parotid gland for disease evaluation, our aim was to investigate the construct validity between these modalities in one and the same gland.MethodsConsecutive sicca patients underwent a multidisciplinary diagnostic workup including parotid gland biopsy, collection of parotid gland–specific saliva and parotid gland ultrasonography. Patients who were classified as pSS according to the ACR-EULAR criteria were included. Construct validity was assessed using Spearman’s correlation coefficients.ResultsThe 41 included pSS patients completed a full workup within a mean time interval of 2.6 months. Correlations between histopathological features and stimulated parotid salivary flow were fair (ρ = −0.123 for focus score and ρ = −0.259 for percentage of CD45+ infiltrate). Likewise, poor correlations were observed between stimulated parotid salivary flow and parotid ultrasonography (ρ = −0.196). Moderate to good associations were found between the histopathological items focus score and the percentage of CD45+ infiltrate, with parotid US scores (total US score: ρ = 0.510 and ρ = 0.560; highest for homogeneity: ρ = 0.574 and ρ = 0.633).ConclusionAlthough pSS-associated ultrasonographic findings did correlate with histopathological features, the three modalities that evaluate salivary gland involvement assess different (or at best partly related) constructs. Therefore histopathology, salivary flow and ultrasonography are complementary measurements and cannot directly replace each other in the workup of pSS.